Cleidocranial Dysplasia - the Jerusalem Approach: part 4

Published: February 2016

Bulletin #52 February 2016

Cleidocranial dysplasia –
the Jerusalem Approach: part 4

Parts 1, 2 and 3 of this series on the treatment of
Cleidocranial dysplasia (CCD), according to the Jerusalem Approach, were
presented in the September, October and November 2013 bulletins on this website
(bulletins #25, 26 and 27 respectively). They describe and illustrate the
rationale for the orthodontic and surgical modality of treatment and its
effective execution in 3 of the 4 distinct stages that are a unique feature of
the Jerusalem approach.

The first stage relates to orthopedic protraction of the under-developed
maxilla,1-3 which may be usefully carried out in those CCD patients
who show a tendency to a class 3 skeletal development, provided they are seen early
enough. The second and third stages are the principal elements in this approach
and they are concerned with extraction of the over-retained deciduous teeth,
surgical elimination of the supernumerary teeth, attachment bonding to the
impacted teeth of the normal series and their assisted eruption and alignment.
1-3

The present bulletin represents the last of the series
describing the comprehensive treatment of CCD according to the Jerusalem
Approach. It addresses the inter-jaw relations that are the result of the late growth
spurt/persistent maxillary under-development or merely exacerbation of the
earlier, frequently-occurring and initially mild, class 3 inter-arch relations.
Most CCD patients are not seen early enough, for a minority the skeletal discrepancy
is too severe, while others may not be adequately compliant with the cumbersome
mechanics involved to benefit from pre-orthodontic skeletal protraction. It is
therefore inevitable that, for a significant number of late adolescents/young
adults who have completed the eruption and alignment of all their permanent
teeth and who display skeletal class 3 relationships, orthognathic surgery will
be indicated.

We have reported that there is a very characteristic 3-4
years delay in dental development in CCD patients in relation to their chronologic
age1-3 and there may also be a lesser degree of delay in the onset
of puberty and circumpubertal growth. For this reason, the timing of the
procedure aimed at surgically harmonizing dysgnathic jaw relations should also
be delayed. The considerations involved in this last phase in the treatment of
CCD by the Jerusalem approach will be illustrated with clinical views of the same
patient described in bulletins #26 and #27.

The incisor-erupting stage began in July 2006. In a
departure from protocol in this particular patient, the eruption and alignment
of the four permanent canines was included in this first stage, together with
the incisors. As the result, this phase of the treatment was particularly long
and was only completed in 34 months, in April 2009. At its conclusion, the 6
maxillary and mandibular anterior teeth had been brought to their place and into
good alignment, although in a marked crossbite relation due to the worsening
skeletal 3 relation. The second tooth-erupting phase involved the premolar
teeth and was initiated in August 2009. Surgery involved the removal of 3
remaining supernumerary teeth in the premolar areas and the exposure and
bonding of eyelets to each of these teeth.

Fig. 1. A periapical view of the right premolar area showing
the “forgotten” supernumerary tooth, at the end of the treatment.

Although it had been identified
prior to the surgery, one supernumerary tooth in the mandibular right premolar
area was forgotten and only re-discovered on the post-surgical panoramic film.
Its presence did not prove to be an obstacle to the successful eruption and
alignment of the adjacent teeth and it was left in situ (Fig. 1).

Fig. 2. Intra-oral views of the occlusion immediately
prior to the orthognathic surgery.

The remaining
orthodontic procedures in this phase were completed in December 2011 with the
angulation of the incisor teeth having been decompensated in line with the
planned post-surgical outcome, thereby producing a very large negative overjet
(Fig. 2).

Shortly after completion, the patient was drafted into the
army, where he spent the next 3 years completing his national service. By the
end of the first year, it was hoped that the final orthosurgical stage of his
treatment would be undertaken during his army service and new records were
taken in February 2013.

Fig. 3. Panoramic view of the dentition prior to the
extraction of the third and fourth maxillary molars.

His orthodontic appliances were left in place and the
maxillary third and fourth (!) permanent molars (Fig. 3) were extracted in October 2014,
in readiness. Circumstances did not permit and the orthognathic
surgery was delayed until his release, in February 2015.

Orthognathic surgery

Fig. 4. Intra-oral views of the dentition a few days
before the surgery, showing the inserted T-pins (Power Pins, TP Orthodontics,
Inc.) to be used as hooks for the intermaxillary fixation.

Surprisingly minimal damage to the orthodontic appliances
was found at the first visit that the patient made following his discharge from
the army and arrangements were made for the jaw surgery to correct his extreme
jaw discrepancy, to bring his teeth into proper occlusion and to improve his
overall facial appearance. In the week prior to the surgery, rectangular
cross-section 0.028”x0.0215” stainless steel archwires were placed and Power Pins
were slotted into the vertical slots of the brackets of all the teeth, to
provide multiple locations for intermaxillary elastic fixation in the immediate
post-operative period (Fig. 4).

Fig. 5. The lateral cephalogram showing the
inter-relations of the hard tissue structures.

The skeletal class 3 relation was due both to
underdevelopment of the maxilla and an over-sized mandible (Fig. 5). In
conference with the surgical team, led by Prof. Nardi Caspi, there was clearly the
need for a 2-jaw procedure, not the least in view of the very large discrepancy
between the jaws. In the final analysis, a LeFort 1 osteotomy was performed
in the maxilla, with a 6mm advancement and four 1.5mm AO plates (DePuy Synthes)
were used for fixation.A bilateral
vertical ramus osteotomy was employed to set back the mandible by 4 mm,
followed by intermaxillary fixation. The chin was treated with a 6mm
advancement genioplasty and fixated with two 1.5 mm AO plates. The patient was
discharged from hospital 7 days after surgery, having been intubated for 2 days
in ICU following respiratory complications during the recovery period.

Fig. 6a. Intra-oral views of the final alignment and
occlusion of the teeth.

He was seen again several times between June and December for
some minor adjustments in my Orthodontic office, before being debonded on 14
December 2015 (Fig. 6a). Fixed 3-3 twistflex bonded retainers were placed in
both jaws on the same day and no other form of retention was used (Fig. 6b).

Post-treatment appraisal

Fig. 7. In the panoramic view, the method of fixation (1.5
mm AO plates)may be seen.

Fig. 8. The post-surgical lateral cephalogram illustrates
the normalization of the skeletal relations and the chin enhancement.

The patient’s overall facial appearance and profile have
undergone marked improvement, both from the point of view of the soft tissues (Fig.
7) and the bony skeleton (Fig. 8). The
face is harmonious and symmetrical, although for reasons of patient
confidentiality, these are not shown here. The upper lip is well supported by
the dentition and there is a good degree of lip competence.

Fig. 9. An excellent relationship between the maxillary
anterior teeth and the lips at rest has been achieved.

The “smile line” of
the incisal edges of the upper teeth in relation to the curvature of the lower
lip is excellent (Fig. 9). Although the pre-surgical records were taken in 2013
(when the patient was 21 years of age) and the immediate post-surgical records
only in 2015 (when he was 23), a comparison of the soft tissue profile is
valid, since growth changes had come to a standstill some time previously.

Fig. 10. Much of the considerable improvement in the
patient’s appearance may be seen in this juxtaposition of the lateral
cephalograms of the patient before and after surgery and “photoshopped” to
bring out the texture of the soft tissues of the face, nose and chin.

Accordingly,
the major clinical benefits that were achieved in the orthosurgical phase of
the treatment (Fig. 10) may be summarized as follows:-

1.Increased lower third of
the face

2.Improved chin profile

3.Improved nasal profile

4.Relatively reduced nasal
length due to the newly supported upper lip and a consequent forward
displacement of soft tissue A-point.

5.Intra-orally, the teeth are
well aligned and the occlusal relations feature good class 1 dental
interdigitation with normal overjet and overbite.

Fig. 11. A periapical view of the splinted maxillary
incisors at the completion of treatment, showing stunted roots and bone loss,
not present elsewhere in the mouth.

.The maxillary central incisors have long clinical crowns due
to a degree of gingival recession (Fig. 6a) and, while the periodontal tissues
are healthy, the panoramic and periapical radiographs show bone loss around the
maxillary incisors (Fig. 11), which may be causally related to the fact that
supernumerary teeth were located on the lingual side of the unerupted incisors
at the outset. A large bony defect had resulted following their surgical
removal. A relative lack of fill-in of new bone during the healing stage,
together with the forced eruption of the labially and superiorly displaced
incisors did not stimulate a sufficiently positive response on the part of the
alveolar bone, to follow the dental development. The left maxillary central and
lateral incisors also have short roots, but before one label this as root resorption,
it is entirely possible that their development was stunted because of their initial
extreme height displacement into close relation with the floor of the nasal
cavity.

Fig. 12a, b. A comparison of the panoramic views pre-treatment
in 2005 and post-treatment in 2015, respectively.

In the mandible, a conscious decision was made not to
disturb the unerupted supernumerary premolar and the unerupted third molars in
the mandible. At this stage, these teeth
cannot adversely affect the orthodontic result and, therefore, the determination
if and when to extract them is no longer an orthodontic decision. The recommendation
was that they should be left in place, monitored clinically and
radiographically on a periodic basis, maintaining the option for their
extraction as and when indicated. Panoramic views of the dentition prior to
treatment and immediately post-treatment can be seen in Fig. 12a and 12b,
respectively.

Fig. 13. Superimposed tracings of the cephalograms (a) May
2005 in black and February 2013 in red, (b) February 2013 in black and January
2016 in red, (c) May 2005 in black and January 2016 in red . Superimpositions by Orthodata, Jerusalem.

Superimposed tracings of the cephalometric superimpositions prior to commencement of treatment (May 2005), at completion of the eruption phase of all the permanent teeth with the exception of third molars (February 2013) and at the completion of the orthognathic surgery phase and removal of all appliances (January 2016) can be seen in Fig. 13a-c. The parameters measured and their values can be read in table 1 below.